Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review

Kong, Steve S; Azarfar, Azin; Bhanusali, Neha

doi:10.1007/s00296-020-04625-1

Cited by 10 publications

(3 citation statements)

References 25 publications

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“…Sneddon's syndrome was considered as the presence of livedo reticularis and stroke. 9 The presence of comorbidities such as smoking, arterial hypertension (defined by blood pressure >140/90 mmHg), diabetes was defined as glucose above 126 mg/dL, and dyslipidemia (defined by National Cholesterol Education Program, 10 as well as the use of medications (corticosteroid, warfarin, antimalarial, statins, acetylsalicylic acid), were also recorded. The warfarin was used to achieve an INR target of 2-3 in venous events and 3-4 for arterial thrombosis.…”

Section: Methodsmentioning

confidence: 99%

Primary antiphospholipid syndrome with and without limb ischemia: A retrospective observational study

de Carvalho,

Brandão Neto,

Skare

et al. 2023

Lupus

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Objective To compare clinical and laboratory data obtained from patients with primary Antiphospholipid Syndrome (PAPS) with and without limb ischemia (LI). Methods A transverse study with 66 (83.3% female) PAPS patients was performed. All data were evaluated. Patients were subdivided into one of two groups: PAPS with LI and PAPS without LI and compared. Results Sixty-six primary APS were selected. PAPS with LI group exhibited a longer disease duration ( p = .012) and more arterial events ( p = .002). A lower frequency of venous events was observed in PAPS with LI ( p = .007), and deep venous thrombosis ( p = .016). Furthermore, PAPS with LI patients had more deficiency of protein C of coagulation ( p = .015) than the others. Conclusion PAPS and LI have a distinct clinical and laboratory spectra from those without LI and it is characterized by an increased frequency of protein C deficiency, and a lower frequency of venous events, deep venous thrombosis and IgM anticardiolipin.

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Section: Methodsmentioning

confidence: 99%

Primary antiphospholipid syndrome with and without limb ischemia: A retrospective observational study

de Carvalho,

Brandão Neto,

Skare

et al. 2023

Lupus

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“…There has also been described an association with valvulopathy, kidney disease, progressive cognitive impairment, and Raynaud's phenomenon. It predominantly affects women (3:1) between 20-42 years [3]. No definite ethnical differences have been encountered [4].…”

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confidence: 99%

“…Antiplatelet therapy or anticoagulation is commonly used. There are some reports considering the use of captopril [3] and immunosuppressants [4] to treat SS, although its benefits have not been established. For primary SS, treatment is not well-defined, with some authors defending antiplatelet monotherapy or captopril as first-line treatment.…”

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confidence: 99%

Sneddon syndrome: a rare cause of stroke hidden in plain sight

et al. 2021

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Successful treatment of Sneddon Syndrome with high dose intravenous immunoglobulin

Reitmajer,

Forchhammer,

Silber

2024

JEADV Clinical Practice

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Sneddon Syndrome is an orphan disease characterized by the clinical presence of livedo racemosa and cerebrovascular ischaemia. Its pathophysiology remains inadequately understood and no validated treatment modality has been established. In this case report of a 37‐year‐old woman, we present high‐dose intravenous immunoglobulin (IVIg) as a potential successful treatment option. The diagnosis was confirmed through a deep spindle biopsy, revealing the typical intima proliferation. Furthermore, we found a positive medical history concerning neurological symptoms. The commonly applied high‐dose anticoagulation had previously not resulted in improvement of the ulcers or pain reduction. The initiated therapy with IVIg significantly alleviated the pain after a single cycle and led to the complete healing of the ulcers 12 weeks later. This case of a patient with subsequent long‐term responsiveness to IVIg supports the approach as a promising therapy alternative.

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Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review

Cited by 10 publications

References 25 publications

Primary antiphospholipid syndrome with and without limb ischemia: A retrospective observational study

Primary antiphospholipid syndrome with and without limb ischemia: A retrospective observational study

Sneddon syndrome: a rare cause of stroke hidden in plain sight

Successful treatment of Sneddon Syndrome with high dose intravenous immunoglobulin

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